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Disaccharidase Deficiency and Malabsorption of Carbohydrates

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SINGAPORE MEDICAL JOURNAL DISACCHARIDASE DEFICIENCY AND MALABSORPTION OF CARBOHYDRATES C K Lee SYNOPSIS A large proportion of man's caloric intake is carbohydrate and starch and sucrose account for over three-quarters of the total consumed. But the rapid change of the food industry from an art to a high specialised industry in recent years have made available a variety of rare food sugars, amongst which are various disacharides. Since the digestion or enzymic breakdown of carbohydrates is a normal initial requirement that precedes their absorption, and metabolism of carbohydrates varies according to their molecular structure, these rare sugars can cause diseases of carbohydrate intolerance and malabsorption. Intolerance and malabsorption can be due to polysaccharide intolerance because of amylase dy- sfunction (caused by the absence of pancreatic amylases) or malfunctioning of absorptive process (caused by damaged or atrophied absorptive mucosa as a result of another primary disease or a variety of other casautive agents or factors, thus resulting in the Department of Chemistry inability of carbohydrate absorption by the alimentary system). A National University of Singapore third type of intolerance is due to primary deficiency or impaired Kent Ridge activity of digestive disaccharidases of the small intestine. The Singapore 0511 physiological significance and the metabolic consequences of such a lactase, sucrase-isomaltase, mal- C K Lee, Ph.D., F.I.F.S.T., C.Chem. F.R.S.C. deficiency or impaired activity of Senior Lecturer tase and trehalase are discussed. 6 VOLUME 25 NO.1 FEBRUARY 19M INTRODUCTION abundance in the free state. It is a constituent of the important plant and animal reserve sugars, starch and Recent years have seen a considerable advance in all glycogen. It arises through the hydrolytic action of saliva facets of enzymology, not least in our understanding of and pancreatic J -amylase (d.-1, 4-glucan 4-glucanohy- the biochemistry, physiological role, nutritional signifi- drolase) on starch. In addition, maltotriose, isomaltose [6- cance, and industrial potential of the enzymes that 0-(d-D-glucopyranose) D-glucopyranose], and some hydrolyse disaccharides, the disaccharidases. Under- branched tri-, tetra-, penta- and hexa-saccharides are also standing of the chemical nature of these enzymes and its produced. A fourth dietary disaccharide is trehalose or relation to catalytic activity has progressed to the point mushroom sugar (d-D-glucopyranosyL. d-D-glucopyra- where the enzymes can increasingly be utilised to advan- noside). This naturally occurring non -reducing sugar is a tage in food and biotechnology processes, analysis and storage sugar of fungi, algae and pteridophytes. In the diagnosis and treatment of gastrointestinal disorder. mushrooms and yeasts, it accounts for as much as 15% of Over the years, with the growth and increased sophisti- dry weight and it can thus be of considerable importance cation of the food industry, significant changes have in single cell protein foods. taken place in the types of carbohydrates that are Food disaccharides, and consequently the disacchari- available in our food supplies. Consequently, various rare dases, the enzymes, that hydrolyse these sugars, have a food sugars are now found in manufactured foods. Since significant role in food and nutrition (1). The physio- the metabolism of carbohydrates varies according to their logical importance of these enzymes is self evident. molecular structure, disorders of carbohydrate intole- Disaccharides cannot be utilised as energy sources rance and malabsorption could arise from the presence of directly, but must first be hydrolysed to their component these sugars. monosaccharides prior to absorption. The metabolic consequences of a deficiency or imparied activity of FOOD DISACCHARIDES disaccharidases are serious and lead inevitably to clinical symptoms such as diarrhoea, vomiting, abdominal dis- Carbohydrates supply a large proportion of man's caloric tension and a general failure to thrive (1,2). However, the needs. In the diets of the poor, especially in the tropics problem of 'fermentative diarrhoea' received little interest they may form as much as 80% of the total calories while in until the later half of the 1950-s when Crane and his the diets of the rich, the proportion is smaller, but still coworkers (3) described a completely new model for the substantial. Much of this carbohydrate is in the di - events occurring during active transport of certain mono- sacharide form, the common ones being sucrose, lactose, saccharides, thereby focusing interest on sugar digestion and maltose, the breakdown product of starch digestion. and absorption in general. These sugars are too large to be absorbed and remains in the small intestine where they are ultimately hydrolysed DISACCHARIDASES to their component monosaccharides by the disacchari- dases located in the brush border of the mucosal epithe- General characteristics lial cells. Sucrose (a-D-glucopyranosy i. R-D-fructofuranoside) The glycosidic bonds are broken by a hydrolytic is widely distributed in the plant Kingdom and is parti- mechanism, involving a transfer of components of the cularly important in food processing. Lactose or milk - substrate to water. The action of carbohydrases generally sugar [4-0-(ß-D-glucopyranosyl) D -glucose] is a natural is highly specific for a particular glycosidic bond linking constituent of milk, occurring either as the free sugar or in particular monosaccharides. Thus, sucrose can be cleav- lactose -containing oligosaccharides. Maltose [4-0-(4-D- ed either by anDC-glucosidase at position (a) or ß-fructo- glucopyranosyt) D -glucose], is not found naturally in furanosidase at (b) (Fig. 1). The configuration, d or ß, Fig. 1 Mechanism of action of carbohydrases HO glucose-[C(1)180H] + fructose (a) OH + H218O OH (b) glucose + fructose-[C(2)18OH] Sucrose 7 SINGAPORE MEDICAL JOURNAL about the anomeric carbon atom is an important factor in pancreatic d -amylase All the oligosaccharides are then determining whether or not the saccharide will be cleaved hydrolysed to the monomeric sugars glucose, galactose by a certain enzyme. For example, both maltose and and fructose by a group of disaccharidases associated cellobiose (4-0-(ß-D-glucopyranose) D-glucopyranose] with the brush border membrane (4). The classification of are disaccharides composed of two molecules of D - brush border enzyme activities (5) is show in Table 1. The glucose. However, cellobiose possesses a ß-glycosidic resulting monosaccharides are actively transported by linkage. Consequently, the two sugars are not cleaved by carrier systems (6,7) [although it was generally believed the same enzyme. The enzymatic hydrolysis of glycosides that fructose absorption is not by an active transport is essentially reversible, although in most cases the system (3)] into the cells and to the portal circulation. hydrolytic reaction is favoured. However, under suitable The disaccharidases of the intestinal mucosa hy- conditions, instead of water, the other monosaccharide drolyse d-glucosidic and ß-glucosidic linkages. The units present may serve as acceptors and lead to the actual activities of these enzymes are shown in Tables 2 formation of new disaccharides or even oligosaccharides. and 3 (8,9). The enzymes are particulate or insoluble The presence of these in significant amounts in foods will enzymes and they are not secreted into the lumen of the undoubtedly lead to metabolic problems. small intestine. Apart from trehalase (d, -trehalose 1-D- glucohyrolase, E.C.3.2.1.28), which hydrolyse only treha- Disaccharidase activities lose, the other enzymes have rather broad substrate specificities. These enzymes exist in isoenzyme forms for Digestion of carbohydrates is initiated in the mouth and which the biological significance has not yet been es- stomach by the action of salivary d -amylase. In the small tablished. intestine, digestion is continued under the influence of Table 1. Disaccharidases in the brush border of the human intestinal mucosa. Enzyme or enzyme complex Substrate Products Lactase-glycosylceramidase Lactose Glucose Galactose Hetero-ß- Galactose galactoside Cel lobiose Glucose Glycosylceramide Monosaccharide N-acylspingosine Sucrase-isomaltase Sucrose Glucose Fructose Maltose Glucose Palatinose Glucose Fructose d --Dextrins Glucose (e.g. isomaltose) Trehalase Trehalose Glucose Two heat stable mallases Maltose Glucose Isomaltose Glucose Glucoamylase (oligosaccharidase) Malto-oligo- Glucose saccharides (2-9 glucose units) 8 VOLUME 25 NO. 1 FEBRUARY 1994 Table 2. Disaccharidase activities in jejuna! mucosal cells (28). Mucosa! protein Disaccharidase activity (per g protein) Subject content (mg/g wet weight) Maltase Isomaltase Sucrase Lactase Normal subjects 1 94 500 123 144 39 2 94 502 110 109 89 3 78 1120 268 325 258 4 330 217 56 49 43 5 150 223 65 70 48 6 147 310 122 105 74 7 91 635 216 229 146 8 105 575 164 167 73 9 112 524 122 143 65 10 72 810 172 195 174 11 111 730 225 138 103 12 130 - 340 87 109 40 Normal subjects with low jejuna! lactase activity 101 86 990 283 255 7 102 103 410 92 115 17 103 112 458 129 139 17 104 91 670 181 188 20 105 130 618 158 234 12 Patients with milk intolerance 201 176 290 93 107 6 202 103 662 204 220 8 204 132 284 88 74 6 Table 3. Actual activities of disacchardases in normal mucosa (9). Activity Enzyme (umol/min/g protein) Maltase 308.8 + 95.2 Sucrase 101.5 + 30.3 Isomaltase 101.6 + 28.4 Lactase 55.8 + 21.9 9 SINGAPORE MEDICAL JOURNAL ENZYME DEFICIENCY
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  • Functional Variants in the Sucrase–Isomaltase Gene Associate With

    Functional Variants in the Sucrase–Isomaltase Gene Associate With

    Gut Online First, published on November 21, 2016 as 10.1136/gutjnl-2016-312456 Neurogastroenterology ORIGINAL ARTICLE Gut: first published as 10.1136/gutjnl-2016-312456 on 21 November 2016. Downloaded from Functional variants in the sucrase–isomaltase gene associate with increased risk of irritable bowel syndrome Maria Henström,1 Lena Diekmann,2 Ferdinando Bonfiglio,1 Fatemeh Hadizadeh,1 Eva-Maria Kuech,2 Maren von Köckritz-Blickwede,2 Louise B Thingholm,3 Tenghao Zheng,1 Ghazaleh Assadi,1 Claudia Dierks,4 Martin Heine,2 Ute Philipp,4 Ottmar Distl,4 Mary E Money,5,6 Meriem Belheouane,7,8 Femke-Anouska Heinsen,3 Joseph Rafter,1 Gerardo Nardone,9 Rosario Cuomo,10 Paolo Usai-Satta,11 Francesca Galeazzi,12 Matteo Neri,13 Susanna Walter,14 Magnus Simrén,15,16 Pontus Karling,17 Bodil Ohlsson,18,19 Peter T Schmidt,20 Greger Lindberg,20 Aldona Dlugosz,20 Lars Agreus,21 Anna Andreasson,21,22 Emeran Mayer,23 John F Baines,7,8 Lars Engstrand,24 Piero Portincasa,25 Massimo Bellini,26 Vincenzo Stanghellini,27 Giovanni Barbara,27 Lin Chang,23 Michael Camilleri,28 Andre Franke,3 Hassan Y Naim,2 Mauro D’Amato1,29,30 ▸ Additional material is ABSTRACT published online only. To view Objective IBS is a common gut disorder of uncertain Significance of this study please visit the journal online (http://dx.doi.org/10.1136/ pathogenesis. Among other factors, genetics and certain gutjnl-2016-312456). foods are proposed to contribute. Congenital sucrase– isomaltase deficiency (CSID) is a rare genetic form of What is already known on this subject? disaccharide malabsorption characterised by diarrhoea, ▸ fi fi IBS shows genetic predisposition, but speci c For numbered af liations see abdominal pain and bloating, which are features end of article.